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Submited on: 19 Sep 2010 02:22:26 PM GMT
Published on: 19 Sep 2010 03:58:08 PM GMT
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Other Comments:
This is a very brief review on the subject better articles on SSPE are available. Instead a illustrative case with video ansd EEG would have been more informative for readers of the areas where SSPE is rare.
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Competing interests:
None
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Invited by the author to review this article? :
No -
Have you previously published on this or a similar topic?:
Yes
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References:
Can be seen in Pubmed -
Experience and credentials in the specific area of science:
I have seen plenty of cases of SSPE.
- How to cite: RK G .subacute sclerosing panencephalitis [Review of the article 'Current trends in the management of subacute sclerosing panencephalitis ' by Arun babu T].WebmedCentral 2011;2(3):WMCRW00553
This is an updating brief review, and as such, serves satisfactorily as an introduction to the subject. The emphasis on treatment is appropriate for the general physician and up-to-date. Some of the spelling and grammar needs editing/refinement. There are no tables.
In countries where measles immunization has not reached 80% of children, suspicion for SSPE should have a low threshhold. Any early decline in behavior, school performance, even appearing like ADHD, should raise Stage 1 SSPE in the differential diagnosis. There should be no problem in clinically recognizing SSPE in Stage II, when periodic myoclonic spasms are obvious. In developed countries a history of early exposure to measles, before measles immunization, in either native or immigrant populations, should also raise the possibility. Unexplained retinopathy, whether discovered incidentally or because symptomatic, may precede clinical Stage I by years. Early diagnosis can also be done by astute clinical examination for periodic myoclonic spasms in someone with mental/behavioral change, by recording an EEG in the standing position, with video if possible, and looking for subtle eye blinks, or loss of posture of extended arms when standing in the Romberg position, coupled with periodic slow wave complexes, The key word is "periodic". One should count the number of seconds between abnormal involuntary movements and see that they are consistently every 10, or 12, or 8 seconds, whatever the period is. The diagnosis specifically is based on clinical history, elevated CSF IgG, and elevated CSF measles antibody titers. The periodic slow wave EEG complexes (they are not epileptiform discharges) early on may appear in sleep only, should be obvious in Stage II, and may disappear in late Stage III and Stage IV.
The review of treatments is brief but comprehsive. The symptoms and signs of SSPE are initially due to inflammatory responses during immunomodulatory treatment, which may slow rate of progression. But chronic progression is inevitable, which may last a decade or more, secondary to atrophy and neuronal loss. So far, no new therapies based on knowledge of the molecular pathophysiology of the disease have emerged.
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I directed the International Consortium on SSPE randomized treatment trial cited in the article, which was published in the Journal of Child Neurology. Prior to that, treated many SSPE patients in Saudi Arabia in the late 1980's, early 1990's, with several publications.